Indian Journal of Radiology Indian Journal of Radiology  

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NEURORADIOLOGY Table of Contents   
Year : 2010  |  Volume : 20  |  Issue : 3  |  Page : 188-191
Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration

Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India

Correspondence Address:
Umesh C Parashari
Department of Radio Diagnosis, C.S.M.M.U. Lucknow
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-3026.69353

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We report a case of a 13-year-old girl with Hallervorden-Spatz disease (HSD) or pantothenate kinase-2 associated neurodegeneration (PKAN). HSD is a rare neurodegenerative disorder, which is characterized by a rapidly progressive extrapyramidal syndrome, dementia with optic atrophy, and retinal degeneration. It is associated with accumulation of cysteine-iron complex in the globus pallidi and substantia nigra. The MRI "eye of the tiger" sign is the characteristic. MRI spectroscopy is also characteristic. It shows markedly decreased NAA/Cr values in the globus pallidi and substantia nigra with increased mI/Cr values that suggest of gliosis.

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